Congenital absence or agenesis of tricuspid valve & inlet portion of right ventricle (RV)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Other Modality Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Lee C et al: Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle. Cardiol Young. 30(1):126-8, 2020
Siddartha CR et al: Tricuspid atresia with truncus arteriosus: off-pump stage I palliation. World J Pediatr Congenit Heart Surg. 10(5):635-7, 2019
Futuki A et al: Absent pulmonary valve syndrome with tricuspid atresia, ventricular septal defect, and aneurysmal dilated pulmonary artery: a case report of successful fontan completion. World J Pediatr Congenit Heart Surg. 9(1):101-4, 2018
Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
Kinoshita M et al: Energetic performance analysis of staged palliative surgery in tricuspid atresia using vector flow mapping. Cardiovasc Ultrasound. 15(1):27, 2017
Alsoufi B et al: Influence of morphology and initial surgical strategy on survival of infants with tricuspid atresia. Ann Thorac Surg. 100(4):1403-9; discussion 1409-10, 2015
Williams-Phillips S: Tricuspid atresia 18 years post Glenn: is Fontan necessary in all cases? West Indian Med J. 64(3), 2015
Rathod RH et al: Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation. J Am Coll Cardiol. 55(16):1721-8, 2010
Choi YH et al: CT findings in unilateral hepatopulmonary syndrome after the Fontan operation. Pediatr Radiol. 39(4):336-42, 2009
Grosse-Wortmann L et al: Aortopulmonary collaterals after bidirectional cavopulmonary connection or Fontan completion: quantification with MRI. Circ Cardiovasc Imaging. 2(3):219-25, 2009
Bhatti S et al: Persistent left superior vena cava (PLSVC) with anomalous left hepatic vein drainage into the right atrium: role of imaging and clinical relevance. Vasc Med. 12(4):319-24, 2007
Sittiwangkul R et al: Outcomes of tricuspid atresia in the Fontan era. Ann Thorac Surg. 77(3):889-94, 2004
Epstein M: Tricuspid atresia. In Allen HD et al: Heart Disease in Infants, Children & Adolescents. Philadelphia: Lippincott Williams & Wilkins. 1197-1215, 2001
Hess J: Long-term problems after cavopulmonary anastomosis: diagnosis and management. Thorac Cardiovasc Surg. 49(2):98-100, 2001
Freedom RM et al: The Fontan procedure: analysis of cohorts and late complications. Cardiol Young. 10(4):307-31, 2000
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References
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Definitions
Congenital absence or agenesis of tricuspid valve & inlet portion of right ventricle (RV)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Other Modality Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Lee C et al: Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle. Cardiol Young. 30(1):126-8, 2020
Siddartha CR et al: Tricuspid atresia with truncus arteriosus: off-pump stage I palliation. World J Pediatr Congenit Heart Surg. 10(5):635-7, 2019
Futuki A et al: Absent pulmonary valve syndrome with tricuspid atresia, ventricular septal defect, and aneurysmal dilated pulmonary artery: a case report of successful fontan completion. World J Pediatr Congenit Heart Surg. 9(1):101-4, 2018
Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
Kinoshita M et al: Energetic performance analysis of staged palliative surgery in tricuspid atresia using vector flow mapping. Cardiovasc Ultrasound. 15(1):27, 2017
Alsoufi B et al: Influence of morphology and initial surgical strategy on survival of infants with tricuspid atresia. Ann Thorac Surg. 100(4):1403-9; discussion 1409-10, 2015
Williams-Phillips S: Tricuspid atresia 18 years post Glenn: is Fontan necessary in all cases? West Indian Med J. 64(3), 2015
Rathod RH et al: Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation. J Am Coll Cardiol. 55(16):1721-8, 2010
Choi YH et al: CT findings in unilateral hepatopulmonary syndrome after the Fontan operation. Pediatr Radiol. 39(4):336-42, 2009
Grosse-Wortmann L et al: Aortopulmonary collaterals after bidirectional cavopulmonary connection or Fontan completion: quantification with MRI. Circ Cardiovasc Imaging. 2(3):219-25, 2009
Bhatti S et al: Persistent left superior vena cava (PLSVC) with anomalous left hepatic vein drainage into the right atrium: role of imaging and clinical relevance. Vasc Med. 12(4):319-24, 2007
Sittiwangkul R et al: Outcomes of tricuspid atresia in the Fontan era. Ann Thorac Surg. 77(3):889-94, 2004
Epstein M: Tricuspid atresia. In Allen HD et al: Heart Disease in Infants, Children & Adolescents. Philadelphia: Lippincott Williams & Wilkins. 1197-1215, 2001
Hess J: Long-term problems after cavopulmonary anastomosis: diagnosis and management. Thorac Cardiovasc Surg. 49(2):98-100, 2001
Freedom RM et al: The Fontan procedure: analysis of cohorts and late complications. Cardiol Young. 10(4):307-31, 2000
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